Diagnosis of Systemic Scleroderma Heart Involvement

Author: Shelley Ensz. Scleroderma is highly variable. See Types of Scleroderma. Read Disclaimer
CT Scans
--ECHO (General)
--Tissue Doppler
Heart Rate
Holter Monitoring


Screening for heart involvement is mandatory for all systemic scleroderma patients.

It is common for patients with systemic scleroderma to develop heart involvement even without any overt cardiac symptoms, so mandatory assessment of systemic scleroderma heart involvement requires a thorough baseline screening and follow-up monitoring. Diagnostic tests for scleroderma heart involvement may include electrocardiograms, echocardiography, Holter monitoring, CT scans, and MRI's. (Also see What is Scleroderma?, Types of Scleroderma, Symptoms of Scleroderma and Overview of Cardiac Involvement)

Study: Unneeded Stress Tests Too Common. Cardiac stress tests done with imaging have risen substantially and more than a third appeared inappropriate. MedPage Today, 10/14/2014.

Six-minute walk test reflects neurohormonal activation and right ventricular function in systemic sclerosis (SSc) patients. Decreased exercise capacity significantly correlates with biochemical and echocardiographic parameters of right ventricular dysfunction and neurohormonal activation providing a potential link for neuroendocrine derangement in patients with SSc. PubMed, Clin Exp Rheumatol, 2013 Feb 13. (Also see Right Ventricular Dysfunction)

Heart involvement in systemic sclerosis: evolving concept and diagnostic methodologies. Echocardiography, including pulsed tissue Doppler echocardiography, is the cornerstone of routine heart assessment. Biological variables, such as B-type natriuretic peptides, are highly relevant, valuable markers of global heart involvement in systemic sclerosis. PubMed, Arch Cardiovasc Dis, [2010].

Diagnostic tests for cardiac problems include Electrocardiogram (ECG) and Holter Monitor. (See Medical Tests)

CT Scans

Usefulness of technetium-99 m sestamibi myocardial perfusion SPECT in detection of cardiovascular involvement in patients with systemic lupus erythematosus or systemic sclerosis. Tc-99m sestamibi SPECT is a useful noninvasive imaging modality to detect cardiac involvement in symptomatic or asymptomatic SLE or SS patients. Lin CC. (PubMed) Int J Cardiol. 92(2-3):157-61.

Echocardiography (ECHO)

ECHO (General) Tissue Doppler Analysis

ECHO (General)

Echocardiogram. An echocardiogram (Also called an echo) is a type of ultrasound test that uses high-pitched sound waves that are sent through a device called a transducer. The device picks up echoes of the sound waves as they bounce off the different parts of your heart. These echoes are turned into moving pictures of your heart that can be seen on a video screen. WebMD.

Aortic regurgitation is common in ankylosing spondylitis - time for routine echocardiography evaluation? As aortic regurgitation and/ or conduction abnormalities might cause insidious symptoms, both electrocardiography and echocardiography evaluation should be part of the routine management of patients with ankylosing spondylitis. PubMed, Am J Med, 06/04/2015. (Also see Ankylosing Spondylitis)

Value of systolic pulmonary arterial pressure as a prognostic factor of death in the systemic sclerosis EUSTAR population. An estimated systolic pulmonary artery pressure >36 mmHg at baseline echocardiography was significantly and independently associated with reduced survival. PubMed, Rheumatology (Oxford), 01/17/2015.

Tissue Doppler Analysis ECHO

Right ventricular systolic dysfunction often precedes the onset of symptoms in systemic sclerosis, however it is not apparent by Tissue Doppler, but can be detected by Strain.

Tissue Doppler ECHO. This test is used to look at how blood flows through the heart chambers, heart valves, and blood vessels. WebMD.

Early Detection of PAH in Systemic Sclerosis Achieved Using Ultrasound Technology. Using right heart catheterization as the gold standard in all patients showed that stress Doppler-echocardiography markedly improved sensitivity in detecting manifest Pulmonary Hypertension. Scleroderma News, 06/22/2015. (Also see Diagnosis of Pulmonary Hypertension)

Prognostic impact of coronary microcirculation abnormalities in systemic sclerosis: a prospective study to evaluate the role of non-invasive tests. Abnormal coronary flow velocity reserve and left ventricular wall motion abnormalities might be related to a worse disease outcome, suggesting a prognostic value of these tests, similar to other myocardial diseases. PubMed, Arthritis Res Ther, 2013 Jan 9;15(1):R8.

Early Detection of Cardiac Involvement in Systemic Sclerosis (SSc) Assessed by Tissue-Doppler Echocardiography: Relationship with Neurohormonal Activation and Endothelial Dysfunction. Depressed cardiac function is common, even in asymptomatic patients with SSc. NT-proBNP and ADMA are significantly correlated with echocardiographic abnormalities, providing a potent link for cardiac function, neuroendocrine derangement, and endothelial dysfunction in patients with SSc who have cardiac disease. Theodoros Dimitroulas. JRheum. March 1, 2010.

Early right ventricular systolic dysfunction in patients with systemic sclerosis without pulmonary hypertension: a Doppler Tissue and Speckle Tracking echocardiography study. IVA is a useful tool with high-predictive power to detect early right ventricular systolic impairment in patients with SSc and without pulmonary hypertension. Cardiovascular Ultrasound, 2010.

Electrocardiogram (ECG or EKG)

Electrocardiogram. An electrocardiogram (EKG or ECG) is a test that checks for problems with the electrical activity of your heart. WebMD.

Heart rate turbulence assessment in systemic sclerosis: the role for the detection of cardiac autonomic nervous system dysfunction. HRT, like HRV assessment, indicates a frequent impairment of the cardiac autonomic nervous system in SSc patients, irrespective of the SSc type. Rheumatology 2010.

Heart Rate

Problem with Heart Rate can be determined with ECG/EKGs and Holter Monitors.

Arrhythmias: Bradycardia and Tachycardia. A slow heart rhythm with a rate below 60 beats per minute at rest is called a bradycardia. Tachycardia is a fast heart rate of more than 100 beats per minute at rest. A fast or slow heart rate does not always mean your heart rhythm is abnormal. Fast or slow heart rates are also related to anxiety, activity, medications or other normal causes. Cleveland Clinic.

Panic attacks. Panic attacks were once dismissed as nerves or stress, but they're now recognized as a real medical condition. Signs and symptoms may include rapid heart rate, sweating, trembling, shortness of breath and hyperventilation, chills, hot flashes, nausea, abdominal cramping, chest pain, headache, dizziness, faintness, tightness in your throat, trouble swallowing, a sense of impending death. Other health problems— such as an impending heart attack, an overactive thyroid gland (hyperthyroidism) or drug withdrawal— can cause similar signs and symptoms. Mayo Clinic. (Also see Lung Involvement, Heart Involvement and Thyroid Disease)


Pseudo–infarction pattern in diffuse systemic sclerosis (dSSc). Evaluation using cardiovascular magnetic resonance (CMR). CMR unveiled that the pattern of myocardial fibrosis in dSSc with Q waves is due to the systemic disease and not to coronary artery disease. PubMed, Int J Cardiol, 2016 Apr 8;214:465-468.

Assessment of myocardial fibrosis and microvascular damage in systemic sclerosis (SSc) by magnetic resonance imaging (MRI) and coronary angiotomography. The prevalence of myocardial fibrosis on MRI attributable to SSc is 45%, is more frequent and severe in diffuse scleroderma, is associated with lower left ventricular ejection fraction and affects mainly basal left ventricular walls. PubMed, Rheumatology (Oxford), 2014 Sep 18.

Diagnostic accuracy of cardiovascular magnetic resonance (CMR) imaging of right ventricular morphology and function in the assessment of suspected pulmonary hypertension (PH) results from the ASPIRE registry. MRI is a robust alternative to echocardiography in the evaluation of patients with suspected PH. Journal of Cardiovascular Magnetic Resonance 2012, 14:40.

Holter Monitoring

What Are Holter and Event Monitors? Holter and event monitors are medical devices that record the heart's electrical activity. They are similar to an EKG, both instead of recording the heart's electrical activity for a few second, they record the electrical activity over several days. National Heart Blood and Lung Institute.

Scleroderma Heart Disease Deaths Might Be Prevented with Heart Monitor. Magnetic resonance imaging (MRI) confirmed rhythm changes in both the upper and lower chambers of the heart, as well as an abnormally fast heart rate and complete heart block which was shown on the heart monitors. Scleroderma News, 06/10/2016.


Scintigraphy may help detect scleroderma cardiac involvement, even before there are heart symptoms.

Definition of Scintigraphy. A diagnostic test that uses radioisotopes. MedicineNet.

Go to Research for Cardiac Involvement
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